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Rheumatology 2001; 40: 102-106
© 2001 British Society for Rheumatology

Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome?

I. Marie, H. Levesque, J. L. Tranvouez1, A. François2, G. Riachi3, N. Cailleux and H. Courtois

Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, 76031 Rouen Cedex,
1 Department of Gastroenterology, Centre Hospitalier Universitaire du Havre, 76083 Le Havre Cedex,
2 Department of Cytology and Pathology, Centre Hospitalier Universitaire de Rouen, 76031 Rouen Cedex and
3 Department of Gastroenterology, Centre Hospitalier Universitaire de Rouen, 76031 Rouen Cedex, France

Objective. We report the cases of two patients with the complete CREST variant (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) of systemic sclerosis (SSc) who developed autoimmune hepatitis.

Results. Our findings suggest that autoimmune hepatitis can be considered to be one of the liver manifestations associated with SSc. Our data also indicate that, because liver involvement may precede skin manifestations, evaluation for SSc is appropriate when autoimmune hepatitis is noted, and that the evaluation should include clinical examination, testing for antinuclear antibodies (especially for anticentromere antibodies) and nailfold capillaroscopy.

Conclusions. From a practical point of view, our two cases emphasize that suspicion of autoimmune hepatitis in SSc patients presenting with cytolytic hepatitis will help to achieve both accurate diagnosis and optimal management.

KEY WORDS: Systemic sclerosis, CREST syndrome, Autoimmune hepatitis.

Correspondence to: I. Marie, 16 rue Arthur Duval, 76300 Sotteville-lès-Rouen, France


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